ABSTRACT
A 35-year-old man has recurrent aseptic meningitis, blurred vision, painful reaction to loud noises, and the skin changes shown here. What is this syndrome called?
a. Achard-Thiers syndrome b. Bamberger-Marie syndrome c. Dejerine-Klumpke syndrome d. Rokitansky-Küster-Hauser syndrome e. Vogt-Koyanagi-Harada syndrome
• Diagnosis requires:
No history of eye trauma or surgery At least 3 of the following:
Chronic bilateral iridocyclitis Posterior uveitis, often associated with retinal detachments
Tinnitus, neck stiffness, cranial nerve palsies, cerebrospinal fluid pleocytosis, or other central nervous system disturbance
Alopecia, poliosis, or vitiligo (face or scalp most commonly)
• In 90% of patients, poliosis or clumps of whitened hair develop in the midst of normal-colored hair. Piebald eyelashes, i.e., clumps of white eyelashes in the midst of normal eyelashes, are shown in this case
• Patients also may have hearing loss, vertigo, dysacusis, headache, drowsiness, nausea, vomiting, dysphagia, and confusion
• Most patients present between the 2nd and 5th decades of life
• Ophthalmologic consultation should be obtained
• Pathogenesis is unknown but is thought to be related to Tcell-mediated autoimmunity to uveal or retinal antigens
• With immunosuppressive treatment, two-thirds of patients obtain vision of 20/40 or better
The condition shown here is associated with which of the following?
